Hemicrania horologica (“clock-like hemicrania”)

Abstract

mg/dL; mean, 58, in 13 of our patients). Finally, he had astonishing root hypertrophy.Hypertrophy of spinal roots, rarely symptomatic, has been occasionally reported in hypertrophic neuropathy (CMT1, Dejerine-Sottas disease, CIDP). 6,7 Most of these patients were not genetically characterized, and the true incidence of root hypertrophy in CMT1A is unknown. Therefore, the mechanism whereby some patients show greater hypertrophy is unclear. In our patient, it is likely that PMP22 tetrasomy was a further stimulus to nerve hypertrophy. Not only were the cauda equina roots hypertrophied but also the ulnar nerves were markedly enlarged, and the biopsied sural nerve showed relevant hypertrophic changes. The onion bulb number was comparable with that of a series of 20 CMT1A patients, 4 but Schwann cell hyperplasia was considerable, indicating the presence of more complex onion bulbs than in almost all these patients. 4 The somewhat rapid deterioration during the past 3 years and high CSF proteins suggested CIDP superimposed on CMT1. However, we found no confirmatory evidence in laboratory, electrophysiologic, and morphologic findings. Root hypertrophy is the likely cause of increased CSF proteins, probably through leakage from enlarged roots and partial block of CSF circulation.

An early 18th-century case description of cluster headache

“…A 35-year-old German female patient described in Johann Christoph Ulrich Oppermann's Dissertatio medica inauguralis de hemicrania horologica (1747) suffered from excruciating daily headache attacks that lasted fifteen minutes. This case of clockwise headache (''hemicrania horologica'') is currently regarded as the earliest account of (chronic) paroxysmal hemicrania by some (21), but not by all (22,23). Additional possible pre-20th-century cases and descriptions of cyclical headaches suggestive of cluster headache were published, among others, by the Italian professor Giovanni Battista Morgagni (1761) (24), the English physicians Robert Whytt (1764) and Marshall Hall (1836) (25), the German neurologists Moritz Heinrich Romberg (1840) (25) and Albert Eulenberg (1871) (26), and self-reported by the German physician Johann Valentin Mu¨ller (1813) (27) and the English vicar Robert Francis Kilvert (1840-1879) (28).…”